Busy ten weeks ahead

One social mediathon, one 20 mile walk, one lobbying trip, one dinner, one craft and yard sale, working with doctors, caregivers, and patients, and daily efforts to recruit walkers and donors for both our Relay for Life team and our Jimmy Fund Marathon Walk team: that’s what my life looks like for the next several weeks in the run-up to the Relay For Life of Greater Fall River June 27-28 at Bishop Connolly High School.

 I know precisely what it means to be a widow or widower.

When I list just the cancer work on my plate like that–and combine it with the usual spring house projects–it is easy to get overwhelmed by everything. But my life has never been less than busy. What makes this tough is facing it–as I have everything since Jane’s death–without her. But that loneliness is a constant reminder of how important this work is to the lives of others.

Raising awareness and cash, Thursday

The heavy lifting for the Walking with Jane 24-in-24 Challenge Social Mediathon on Thursday has already begun. Putting together the ideas and materials for 12 hours of programming takes some time and some thought. The good news is that others have already done much of the video work that needs to be done and it is a simple matter of collecting those materials from the appropriate sources.

…my life has never been less than busy.

Unlike, however, our November Zebra Social Mediathon, this effort is also connected with trying to raise a substantial chunk of money. The goal this year is to raise $2400 in 24 hours. Last year, that was the goal as well. But we only managed about $1600 with that effort compared to the $2400 we raised the previous year with a simple email campaign. All the money we raise will benefit our Walking with Jane Relay for Life of Greater Fall River team.

Talking to politicians, recruiting sports teams

April 30, I’ll be in Boston to talk with state senator Mike Rodrigues about a palliative care bill the American Cancer Society Cancer Action Network is backing in the Massachusetts legislature. I’ll also be talking with him about getting a proclamation issued making November NET Cancer Awareness Month again this year. I want to get that done early so that we can work with several other groups to make NET Cancer Awareness Month much more broadly acknowledged by real people in the Commonwealth and elsewhere.

The goal this year is to raise $2400 in 24 hours.

A number of us are hoping to get all our professional sports teams in New England to put a zebra ribbon on their uniforms in November. That will require dealing with the various leagues as well as the teams. Having that proclamation in hand will make that a bit easier. We’re also trying to get a formal presentation of the proclamation with a truckload of dignitaries, patients and caregivers in attendance so that we can get the media attention we need to really make people aware of carcinoid/NETs.

Teaming up with ADVOCATS and the Grange

May 22, we will help the Westport High School ADVOCATS put on a clam cake and chowder dinner at the WHS cafeteria in Westport, MA. The last two years we did a spaghetti dinner that did not do as well as we would have liked last year. Depending on how the event goes, we may give up on dinners in the future. This event also supports our Walking with Jane Relay for Life of Greater Fall River team. Tickets are $12 per person.

…we need to really make people aware of carcinoid/NETs.

Just over a week later, on June 1, we’ll host our Third Annual Walking with Jane Yard & Craft Sale at the Westport Grange Hall. Admission is free. Vendors pay $25 for an indoor or outdoor site–all of which goes to our Walking with Jane Relay for Life of Greater Fall River team. We already have six people interested in joining us with things to sell, and I just posted the event broadly yesterday.

Walking the Walk–literally

I’ll personally be doing a lot of walking between now and the Relay For Life. May 4, I’ll tackle the 20 miles of the Walk for Hunger. May 17 I travel to Chelmsford for the Cystic Fibrosis Walk. And every day I’ll put in at least five miles–and often 10–as I train to spend the night on the track June 27-28.

… I just posted the event broadly yesterday.

Killing cancer, like beating hunger or cystic fibrosis or MS, takes lots of money and lots of effort. But that effort is as nothing to the effort that patients and their families face. I can’t tell you what it is like to be a patient. I can tell you what it takes to be a caregiver and a spouse. And I know precisely what it means to be a widow or widower. I live that life every day. I want no one else to face that life as a result of cancer.

The search for the Holy Grail

One of the holy grails in carcinoid/NETs research is a reliable diagnostic test that will consistently find the presence of the disease. Many forms of imaging, blood tests and urine tests fail to see tumors that a surgeon’s knife discovers. But opening up a patient absent a good reason to do so is not a practice any reputable surgeon would consider.

…it could be a game changer…

As a result, many patients are excited by a new test, called NETest, marketed by Wren Laboratories that promises to detect carcinoid/NETs tumors with a single blood test. The test is based on three papers by researchers from Yale and uses 51 different genetic markers that, taken together, indicate the presence of carcinoid NETs in a person’s body according to the researchers.

Claims for the test

According to the company website, the NETest can reliably tell if a patient has carcinoid/NETs. It says it can be used after an operation to determine whether or not surgeons got all the tumors there were to get or not. It says it can measure the efficacy of treatment. It says it can be used after a “cure” to determine whether or not the disease is really entirely gone.  Finally, it says it can, if you have symptoms, determine whether or not those symptoms are caused by carcinoid/NETs.

…many patients are excited by a new test…

The test uses two five ml blood samples and does a polymerase chain reaction test on them to unveil those 51 genetic markers that may be hiding in the sample. The lab then reports those results on a 0-8 scale with 1-4 representing low levels of NETs activity, 5-6 representing medium activity, and 7-8 high activity. Both the site and the papers the test is based on say the test has a high sensitivity level for carcinoid/NETs: 85-98 percent compared to the 64 percent recorded for the Chromogranin A test that is currently in use. CgA tests for a single peptide many–but not all–carcinoid NETs tumors produce. The company says the better  results come from the fact it is looking for 51 markers that are all associated with the disease.

The cost of the test

While the samples themselves do require some special handling, there are no real restrictions on what foods patients can have beforehand–nor much of anything else. The cost of the test is $750. If you want a consultation with one of their physicians before or after the test, it’s another $1000–but that consultation is not required.

The test uses two five ml blood samples…

However, the test is not yet covered by insurance. The company says they have applied to the AMA for a billing number and their decision is due this month (April 2014). Meanwhile, they are offering three free tests per patient. So what’s not to like?

Where is the FDA testing?

The papers the test is based on were written last fall. The test became available very quickly after those papers were published. That struck me as peculiar because most tests don’t become available until they have gone through a series of FDA trials. Even a Phase I trial for an orphan disease like carcinoid/NETs takes more than six months to complete. I first heard about a possible blood test to replace the 5-HIAA urine test not long after Jane’s death–and it may have been around well before that. It is only becoming available broadly now.

…the test is not yet covered by insurance.

I can find no record of an FDA trial for NETest. The website says the test has been validated with over 600 NET cancer patients–and another 1000 either cancer free or with other cancers. But if that took place under an FDA regimen, I would expect that to be mentioned. It isn’t. The only trials specifically mentioned are going on overseas.

What the numbers say–Part I

In the company’s defense, they do say the test is being used at “the Mount Sinai Hospital (New York), the Mayo Clinic (Rochester), and Yale University (New Haven) and is being implemented at Cedars-Sinai Medical Center (Los Angeles), the University of Iowa (Iowa City) and Columbia University (New York),” all of which are respected cancer centers in the US. But the tests were also available at a number of patient conferences as well–bypassing traditional ways of gaining access to patients.

I can find no record of an FDA trial for this test.

There are also some problems involving the way in which one of the papers lays out the basics of carcinoid/NETs incidence and prevalence. Using the same statistics every other paper uses to declare this a rare disease–an incidence of 3.6/100,000 and a prevalence of 35/100,000–the authors declare the disease common. I am not quite sure how that works.

What the numbers say–Part II

Do not misunderstand me: I think the disease is significantly more frequent than those numbers show. But I can muster no evidence beyond the anecdotal for that belief. Until we have other, concrete and replicable, evidence to dispute the prevalent view, we don’t get to argue the disease is common on the scientific stage. Nor do we get to cite the same numbers that declare the disease rare to declare it common.

…the authors declare the disease common.

That use of the interpretation of numbers makes me nervous about the rest of the paper: is this a work of science or is it the prospectus for a business venture? The two don’t mix very well for me.

Financial Connections

Another problem is the connection between Wren Laboratories and two of the authors of the papers who have a financial connection to the company. Irvin Modlin, the Medical Director of Wren Laboratories, is the primary author on two papers the test is based on, and the second author of a third paper cited on the website. Mark Kidd is the company’s Scientific Adviser–and the third author on two papers.

 Nor do we get to cite the same numbers that declare the disease rare to declare it common.

The authors do acknowledge in the studies that the research was funded by Clifton Life Sciences, but make no mention of Wren in their disclosures. Perhaps that is because Wren was only formed after the pieces went to press, but some acknowledgement on their website of that connection for papers posted there would be the ethical thing to do–or so it would seem to me.

The scientific method

That connection may not mean much to some people, but it evidences a significant problem: there is no independent acknowledgement that what they are saying is true. No one has independently reproduced their results–and that is one of the hallmarks of modern science: can someone else replicate your results independently of your lab and supervision?

 …two of the authors…have a financial connection to the company.

I am not saying they are lying or that they fudged their results. But I grew up in a household where the scientific method was sacred–and part of that method is independent verification. That seems to be missing here. I’d be much more comfortable if it were present. FDA trials would provide that.

Where do we go from here?

For now, at least, we need to treat these results as preliminary, for all that they are interesting–even promising. I can fully understand the excitement patients feel about them. I feel that same excitement myself. And for those who have been trying–sometimes for years–to get a straight answer about what they have, the test may offer that knowledge.

No one has independently reproduced their results…

But from a medical point of view, there are other questions that need to be answered before doctors can be entirely comfortable with it. In addition to those I’ve raised above, these include how–or if–this test should influence patient treatment; who should be tested and when; and finally, what ethical questions will doctors have to deal with? Assuming the test does work as advertised, these pieces of the puzzle still need to be addressed.

Trust, but verify

I really hope that this  turns out to be the armor-piercing bullet we are all looking for. It would be nice to have an ironclad diagnostic tool for carcinoid/NETs. It would be more than nice: for many, many patients it could be a game-changer. It could be a game-changer for researchers as well because it potentially provides a fuller genetic map to the disease’s weaknesses.

I feel that same excitement myself.

But until these red flags are lowered, I am not entirely willing to place even a small wager on what is on offer here. After the three free tests, each additional test is $750. Whether insurance companies will agree to pick up that tab absent FDA approval or other replication of the results remains to be seen.

Another person diagnosed with Jane’s cancer

Every day, 33 people are diagnosed with carcinoid/NETs. Every day 33 people with that diagnosis die from the disease and its complications. Most live for several years–even decades–between the two events. Others, like Jane, for a variety of reasons, are gone in what seems like a heartbeat.

 You face a real struggle in dealing with this disease, but there is hope.

A friend was diagnosed with carcinoid/NETs in recent weeks. I only learned about it this weekend–and when I did it brought back all the memories of our first weeks dealing with the disease in 2010. I wish we’d known then what I know now. But so much of what I wish we’d known did not exist then.

Newly diagnosed need to learn the basics

That–and some posts by people newly diagnosed with carcinoid/NETs on the various online support groups–got me thinking about the need for a primer for patients on what resources are available and background materials. Sites like this one have lots of information about the disease, but in the panicky first weeks after diagnosis no one is in a mental state to sift through  dozens of sites trying to figure out what is useful and what isn’t.

I wish we’d known then what I know now.

I quickly put together a note to my friend about the things I thought she needed to know–the things I wish we had known at the beginning. What follows is based on what I told her. If you are recently diagnosed, it may prove useful to you, as well.

The moments after diagnosis are scary

First, take a deep breath. You are probably feeling a thousand different emotions this point–and your mind is likely chasing off in an equal number of directions. Your doctor has likely told you this is a rare disease–may even have told you he has never seen a case of it before. Your oncologist has likely told you something similar. Those are scary things to hear from the people you view as the experts you have put in charge of your health.

Sites like this one have lots of information…

We don’t train doctors to deal with unusual diseases very well. They are taught from the beginning of medical school that when they hear hoofbeats they should think horses, not zebras. What that means is that a particular set of symptoms most likely is the result of the most common illnesses (horses) associated with those symptoms, not some other disease that is relatively rare (zebras).

The newly diagnosed zebra

Carcinoid/NETs is a zebra even in the cancer community. We diagnose about 12,000 cases in the US every year. There are about 120,000 people living in this country who have been diagnosed with it at any given time. Both those numbers have been rising in recent years, though whether that is because there are more people getting it or whether our ability to detect it has gotten better is unclear. What is clear is that about 12,000 people who know they have the disease die of it every year.

First, take a deep breath.

If your cancer was detected early, there is a good chance surgery will cure you. Unfortunately, this cancer is rarely detected early. If the cancer has spread beyond the primary site, we currently have no cure for the disease. We do have  therapies that will often slow it down and alleviate some of the symptoms. And we do have some things in trials that offer the possibility of a cure for at least some patients.

Newly diagnosed have reasons for hope

In most cases, this is a fairly slow-growing cancer. The therapies we do have may buy you enough time and quality of life for better therapies to come along. Things are changing rapidly in this field and you have reason for hope.

Carcinoid/NETs is a zebra even in the cancer community.

Carcinoid/NETs is unlike any cancer you have heard of before. Most tumor cancers take up space and consume resources. Eventually, they are consuming more resources than your body has or begin to impinge of the spaces of vital organs and destroy their functions.

This cancer is radically different

The primary tumor for most carcinoids is very small–often getting no larger than a lentil. The daughter cancers–the metastases–may get much larger, especially those in the liver, but even those are harder to detect than the more normal cancers. Regular scans often fail to pick up even the larger carcinoid tumors. Every tumor has what are called receptors on them. Contrasting agents–essentially radioactive dyes–are picked up by those receptors, making the cancer visible.

Things are changing rapidly…

But carcinoid tumors lack the common receptors so the dyes don’t stick to them–sort of like the wax on the Easter eggs refusing to pick up the color. This helps keep the tumors seemingly invisible. Two new types of scan can see carcinoid/NETs tumors by using radioactive agents that are traced by their decay —octreoscan and Gallium-68. Unfortunately, some carcinoid tumors are not revealed by those either–and remain invisible.

But just because you can’t see them doesn’t mean they are not there.

The hormone/peptide difference

Another difference between this form of cancer and others is that these tumors can–and often do–produce any of the more than 24,000 hormones and peptides the body produces. Increasing the amount of any hormone can have a huge impact on your life because each is involved in body chemistry.

…just because you can’t see them doesn’t mean they are not there.

For example, my wife’s tumors were producing serotonin. Serotonin is involved in controlling digestion, sleep, respiration and blood pressure among other things. Too much of it results in food being digested too quickly for the body to absorb its nutrients–and results in frequent diarrhea; in greater and greater difficulty falling asleep and staying asleep; in dangerously low blood pressure and respiration.

Diagnosing hormone issues

For a long time we had only one test for excess serotonin levels: the 5-hiaa urine test, which requires the patient collect all their urine over a 24 hour period. It is a cumbersome test and doctors, not wanting to put patients through it, can be slow to order it–especially when the disease it tests for is rare. A blood test for excess serotonin appears to have finished trials and is now available, but its broad use is still some years away because few doctors know about it.

Increasing the amount of any hormone can have a huge impact…

But the hormone or peptide being produced doesn’t have to be serotonin–it’s just the one we check for most often. It can be any of those 24,000+ hormones and peptides–and we only have tests for a relative handful of them. And the result of those excess hormones can also result in carcinoid syndrome.

Unique tumor biology complicates life

This complex biology is the reason a mainstream oncologist is often not the best choice for dealing with this disease. I can’t tell you how often I’ve heard things like, “Your 5-HIAA urine test came back negative, so we can rule out carcinoid/NETs;” or “The scans all came back negative, so it can’t be cancer.” The doctors involved were excellent doctors. Their lack of experience with this particular cancer left them prone to mistakes.

…we only have tests for a relative handful of them.

I can’t say strongly enough how important it is to find an oncologist who is familiar with this disease and who can put together the right team for you. Jane and I were lucky to have two of the best people in the field virtually in our back yard–and a local oncologist knowledgeable enough to send us to them.

Medical resources for newly diagnosed

There is a list on our resources page of some of the places that have carcinoid centers at them. That list is not exhaustive, however, because new centers pop up with some frequency. Nor can I recommend any of them, save one, based on personal experience because I have not worked with them. They are often worth the trip because the doctors working at them should have both the expertise and the resources available to make things the best they can be for you. There are also many fine individual doctors with experience with this disease outside those centers. The Carcinoid Cancer Foundation maintains an extensive list of doctors who report they have expertise in this area.

Jane and I were lucky to have two of the best people in the field…

That specialist should put together a team of specialists in other medical areas your cancer will have an impact on. In Jane’s case, because the serotonin was affecting her heart, we also had an oncocardiologist and a heart surgeon on our team–and would have added a liver specialist in due time if she had lived.

Support resources for the newly diagnosed

Just as important as finding the right team of doctors is finding others with the disease to talk to. When Jane was diagnosed, the nearest group we could have found was in New Jersey. Honestly, even had we found the New England group I discovered 18 months after Jane’s death, their meetings would have been too far away for her to get to.

There are also many fine individual doctors with experience…

Now, several groups have moved online. There are several support groups on Facebook who function very well. They are private groups open only to patients and caregivers, but all you need to do is ask to be admitted. All the groups below are useful but each has a slightly different personality. I spend time in each regularly, trying to be useful for both caregivers and patients alike.

Carcinoid Cancer Awareness Network

Carcinoid Coffee Cafe

Carcinoid Neuroendocrine Cancer Awareness

ZEBRA Awareness Global NETS

Lovable Lungnoids  (This group deals specifically with carcinoid lung cancer)

The Carcinoid Cancer Foundation also maintains an extensive list of support groups.

Information resources for the newly diagnosed

You can also find additional information and support though a number of other organizations. First, walkingwithjane.org is the website of Walking with Jane, the foundation I set up shortly after Jane’s death to raise money for research and raise awareness about carcinoid/NETs. It has lots of information, but is also in the midst of a renovation project, so I am a bit behind on some things. Use its Resources section to find other groups and organizations, as well as some basic information about the disease. The News Archive at bottom of every page may also prove useful to you.

…several groups have moved online.

The organization that has dealt with this the longest is the Carcinoid Cancer Foundation. Their site has lists of doctors who have dealt with the disease before, as well as national and regional support groups. The Caring for Carcinoid Foundation in Boston has similar, though less extensive, listings. They have a strong research focus.

There are many good videos online the Carcinoid Cancer Foundation has posted–including all the sessions from some excellent patient conferences. Walking with Jane has also posted some, though our collection is dwarfed by theirs. Here is a basic FAQ video we did last fall.

Some final thoughts

I hope this has helped you get through some of the initial shock of being diagnosed. Things are much better now than they were four years ago when we were just beginning to try to figure out what was going on with Jane. There are some therapies in the pipeline that offer hope of a complete cure for many patients. And our increasing knowledge of how this beastie works offers hope of even better treatments in the near future.

There are many good videos online…

Be optimistic, then. You face a real struggle in dealing with this disease, but there is hope. You will often have to be your own advocate, but the doctors you will need to find will understand that sometimes you will have heard something before they have. One of the standard openings at patient conferences is that anyone who has dealt with this disease for more than six months has the equivalent of a Ph.d in the subject. There is more than a little truth in that statement.

Editor’s note: None of the above constitutes medical advice. I am not a doctor–and don’t pretend to be. The information above is based on my experience dealing with my wife’s battle with carcinoid/NETs and what I have learned since from talking with her doctors, other patients and caregivers, and reading everything I can find on the subject. Before making any medical decision you should speak with your doctor about your particular situation and circumstances. They are qualified to help you make decisions on these things. I am not.

Overwhelmed by carcinoid/NETs stories

I am feeling a bit overwhelmed the last few days. I’ve spent some time on the sites of five different carcinoid support groups. Their experiences are jarring. Not only do they bring back memories of Jane’s struggle, but they underline just how lucky Jane and I were to have access to the doctors and resources in this part of the country.

We have to keep working to find answers…

We did not look forward to the 90-120 minute commute each way into Boston. That trip is a walk in the park compared to what most patients face. Several are traveling hundreds of miles to see the kinds of specialists we take for granted. Those who do not travel those kinds of distances often have to deal with doctors who have little or no experience with this most slippery of all cancers.

Overwhelmed by confusion and frustration

Those doctors do the best they can under the circumstances. But is it any wonder that patients become frustrated and confused when their doctors’ own frustrations and confusions are on display. I’ve read twice today alone of patients whose doctors were mystified that the patients’ tumors had gotten smaller after Sandostatin injections. I can’t count the number of times doctors have told patients–just this week–that because their 5-HIAA urine tests were negative they could not possibly have carcinoid.

…those savants are not always easy to get to physically…

In the first case, there is nothing miraculous about carcinoid tumors sometimes shrinking because of Sandostatin or Octreotide treatments. In a small number of cases, the tumors do actually shrink. The drugs block a receptor on the surface of the tumors. If the tumors happen to be just the right type, they starve a little as a result, and shrink. More often, the tumors stop growing for a while. Too often, though, the tumors continue to grow and spread despite the treatment.

Overwhelmed by lack of information

Some of the drugs in trials at the moment have more success than Sandostatin and Octreotide in shrinking and stopping tumor growth. The CAPTEM combination reported on in January has even recorded some complete remissions–as well as shrinking and delaying tumor growth, according to the interim report on a very small Phase II study. But no therapy we have under study works on everyone.

…we have no single test that can absolutely rule carcinoid/NETs out.

Over 60 percent of all the carcinoid/NETs we diagnose involve the production of serotonin. The by-products created when the liver metabolizes serotonin are what the 5-HIAA urine test is designed to detect. About 35 percent of the carcinoid/NETs diagnosed involve the digestive hormones and peptides created by the pancreas. They require a different set of tests.

Overwhelmed by complexity of diagnosis

The body produces over 24,000 different hormones and peptides–and carcinoid/NETs can produce any of them. Unfortunately, we only have tests for a relative handful of those hormones and peptides. And even the tests we have must be ordered by the physician to be of any use in diagnosing the disease.

…no therapy we have under study works on everyone.

The problem is we have no single test that can absolutely rule carcinoid/NETs out. Even the much-praised Gallium-68 scan–which is still in trials–and the Octreoscan patients and doctors have begun to rely on cannot be counted on to detect the disease. If the tumors have a different variety of receptor that does not take up the contrast agent, those scans will be just as useless as any of the traditional scans doctors have used to detect other forms of cancer.

Overwhelmed by the search

Dr. Eric Liu posted on Facebook last month about the value of having a carcinoid/NETs specialist who has seen a significant number of carcinoid/NETs patients. It is one thing to have read about the disease amid all the other things even a general oncologist has to read to stay current on the more common cancers. It is something else entirely to have made carcinoid/NETs the focus of your work. It is one thing to have treated one or two patients with this oddball cancer. It is something else entirely to have worked with hundreds–or even thousands–of people afflicted with the disease.

…doctors do the best they can under the circumstances.

But those savants are not always easy to get to physically–let alone find. Insurance companies continue to give people grief–even under the new regimen–about going “out of network” or wanting expensive drugs that will decrease the company’s bottom line. There are endless hurdles patients have to face–along with the debilitating symptoms.

Resolving confusion, resolving frustration

The size and scale of the problems carcinoid/NETs patients face every day can overwhelm them. We can’t let that happen. We have to keep working to find answers that will not only save their lives, but also give them a better quality of life.

I am feeling a bit overwhelmed the last few days.

That begins with educating both primary care doctors and traditional oncologists about what our  current tests can and cannot do–and why they need to be careful about the conclusions they draw from those tests we do have. It also means finding better ways to let both doctors and patients stay up-to-date about treatments for this perplexing beast of a cancer.

Last words to the dying

I spoke to my conscious wife for the last time 40 months ago today. She came out of the coma she had entered shortly after noon at 5:57 p.m. She could not talk.  I told her the doctors said there was nothing left to try and that we would take her off life support the next morning.

…one of the more horrible arrows in Death’s quiver.

She shed a single tear when I told her that. I told her I loved her. I told her I was sorry I had failed her. I told her she was going home to the garden we both came out of more than half a century before. I told her I loved her. She closed her eyes. I kissed her forehead and whispered, “Good night, my warrior princess.” I held her hand until the nurses insisted I go find something to eat. I came back and held her hand until they told me to go to sleep.

Descent into death

Telling her she was going to die may seem cruel to some people. But Jane prized honesty above everything. She needed to know what was happening. The greater cruelty would have been to lie to her, given who she was and what we both believe.

…there was nothing left to try…

In the morning, I read to her, chanted to her, sang to her the songs of our faith. I talked to her. I held her hand as they removed the feeding tube, turned off the oxygen, and took the sensors and IVs away. I descended into death with her as far as a living man can. Just before 8 p.m., there was a catch in her breath. I leaned in to kiss her and caught her final breath with my lips.

Forty months in my personal wilderness

Anyone who wants to understand English literature has to understand the symbolism involved in colors and numbers. Forty is the number associated with trials and temptations. Moses and the Israelites spent 40 years in the desert, Christ spent 40 days in the wilderness.

I descended into death with her…

Forty months after Jane’s death I am beginning to ascend from the gates of Death where I left Jane. Some days, I feel almost human–almost alive. Then, suddenly, I am back in that hospital room being brave and strong despite the certain knowledge that everything had unravelled in less than 24 hours and there was nothing more I could do than hold Jane’s hand and help her let go of the shattered vessel that had been her home.

Forty months of battle, forty months of healing

Forty months after Jane’s death I can finally move my wedding ring from my left hand to a finger on my right for a few hours each day. Last night, I did that for the first time in a public setting. I am finally able–for short periods of time–to accept that the “until death do us part” has happened and that my marriage to this extraordinary woman is over.

Then, suddenly, I am back in that hospital room…

Forty months after Jane’s death, though, my personal battle against the disease that took her life–that ended our life together–is far from over. We know more about her disease than we did on that August morning when her doctor told us what she had. We know more about it than we did the first time we met with Jennifer Chan in Boston. We know more about it than we did the night Jane killed it in the only way anyone ever has whose disease was not discovered before it could be detected.

Forty months of good and bad

Forty months after Jane’s death, we still have no cure. We can still only alleviate the symptoms some of the time–though we have a couple of new ways to do that. People still sit in their bedrooms or bathrooms injecting themselves in the belly once or twice or three or more times a day.

We know more about her disease than we did…

But 40 months after Jane’s death we are spending three times as much on research than we were spending when she died. Forty months after Jane’s death we have a couple of things that look like they have the potential to turn into a cure for at least some patients.

Forty months of small victories

Forty months after Jane’s death we have some better–though not perfect–means of detecting the disease. Forty months after Jane’s death, we have some small embers hope. Whether those embers can be coaxed into a full flame remains to be seen. These are difficult times financially–and research costs money.

…we still have no cure.

Forty months after Jane’s death I spend my days trying to help find the answers to this disease–and to bring comfort to those who are afflicted with it. I read, I think, I write, I plan. I work to raise awareness and spread the knowledge we have gained in those 40 months. I work to help raise the money to fund the research that will tell us the things we need to learn in order to find a cure.

Forty months of healing

But forty months after Jane’s death I also spend a part of every day trying to heal the deep wounds losing her has inflicted on me. Watching the person you love most dying in front of your eyes is a shattering experience. It is an experience I would shield Satan himself from.

…we have some small embers hope.

Carcinoid/NETs is one of the more horrible arrows in Death’s quiver. My work now is to steal that arrow so that no one lives the death Jane faced.

When is a zebra not a zebra?

Most people think of zebras as beautiful animals that inhabit the plains of Africa. They feel badly when a lion or some other predator drags them down. They are harmless animals: wild horses with wonderful stripes painted on their bodies.

There is nothing cute about this disease.

The night before Jane died, her heart surgeon and I had a conversation in the hall about Jane’s struggle with carcinoid/NETs. He explained to me the adage young doctors are taught: “When you hear hoofbeats, think horses not zebras.” It exists to remind them that the most common answer to a set of symptoms is most often the right answer.

The zebra as predator

Unfortunately, when the disease actually is rare, looking for horses is exactly the wrong approach. It can–and often does–lead to death.

Most people think of zebras as beautiful animals…

“A zebra killed your wife,” the surgeon said to me. He was not cold when he said it. Jane’s death hurt him. I could see that. He had done everything he could–everything he knew how to do–to preserve her life. And he had failed. He was the kind of doctor who does not like to fail–not because of ego, though those people exist–because failure means a life gone too soon, a life ended that still had things to offer the world.

The zebra as sadistic killer

I’ve never been able to think of zebras in the same way since that night. A zebra spent 30 years kicking my wife to death. It gnawed on her guts more insidiously than any lion ever devoured a zebra. A lion kills its prey before dining. This zebra had delighted in eating her while she was still alive–rejoiced in every diarrhea bout it induced, in every sleepless night, in every painful moment created by a belly filled with gas.

‘A zebra killed your wife.’

The ancient Greeks had a tradition of calling truly hideous things that made them appear less gruesome than they were. The creatures we call the Furies–goddesses charged with punishing patricides and matricides–they referred to as the “Libation Pourers.”

Owning the zebra

Those who suffer carcinoid/NETs have done a similar thing. They have made their emblem the zebra. They call themselves zebras and refer to their support groups as zebra herds. Some even collect stuffed zebras. They wear the word almost as a badge of honor.

A lion kills its prey before dining. 

And it helps. It helps them forget about the constant diarrhea that makes any trip into new terrain an adventure. It helps them forget that they know where the bathrooms are in any store or mall they frequent–and exactly how long it will take them to get there. It helps them mask the fact this hideous zebra is slowly devouring their lives–is kicking them to death one tumor at a time.

Another long good-bye

I understand it–and I hate it. To the rest of the world, zebras are cute. To me–and to anyone who has dealt with carcinoid/NETs–they are the most dangerous and insidious of predators. They are perfectly disguised for their trade. They are more hideous than the cheetahs of the cancer community–the cancers that kill swiftly and with comparatively minimal fuss.

…kicking them to death one tumor at a time.

If Alzheimer’s is the long good-bye, carcinoid/NETs is its cancer equivalent. It takes its time killing its prey–and leaves the prey completely aware of the pain and suffering it is creating.

Hunting the zebra

People who have not seen the disease up close don’t understand. Some doctors tell their newly diagnosed patients about its slow growth habit as though that were a good thing. Those of us who have seen it know that slow growth habit makes it harder to kill, harder to diagnose, harder to find–harder in so many ways–and ever so much more dangerous than some of its quicker-footed cousins.

It takes its time killing its prey…

There is nothing cute about this disease. It is a wolf dressed up as a zebra. And every day it ends the lives of 33 more members of the herd. It’s time to hunt it down and kill it.

Research: Drug/Radiation combination works in mice

A combination of an oral cancer

drug that inhibits the VGEF pathway that stimulates blood vessel growth in tumors and targeted internal radiotherapy reduced tumor volume by 97 percent in transgenic mice with pancreatic NETs, according to an article in EJNMMI Research.

The group combined an oral dose of vatalanib with the radio-peptide [lys 40(Ahx-DTPA-111ln)NH2]-exendin-4, that binds with the GLP-1R receptor on insulinomas and other NETs. The combination worked better than either treatment alone.

Drug reduces radiation dose

The valatanib, however, allowed for a much smaller dose of radiation, resulting in no damage to the pancreas. The valatinib had to be given at about the same time as the radiation because giving it earlier decreased the amount of radiation the tumor absorbed.

Use in humans is a long way off, of course. The technique will face the lengthy drug trial process before becoming broadly available. Many treatments that work in animal studies prove not to work at all in people.

Diagnostic technique wins orphan status in Europe

announced its OPS202–a gallium-68 driven PET scan–has received an orphan drug designation from the European Medicines Agency.

According to the preliminary research results released at the ASCO conference in San Francisco, the gallium-68 scan has “shown superior results compared to other modalities.” While the scan is diagnostic, it has therapeutic possibilities as well, using Yttrium-90 (90Y)–which is also under study.

Research into method taking place in US, Europe

The diagnostic procedure, which will go by the trade name SOMscan, takes about an hour to do. The gallium-68 binds to the somatostatin receptor subtype 2 on the surface of the carcinoid/NETs tumor.

Phase I trials will start in Europe later this year. A Phase II trial will start in the US at Vanderbilt this year, as well.

Tumor profiling aids treatment

Caris Life Sciences presented on its comprehensive tumor profiling service at the San Francisco ASCO meeting. They said their profiling program found drug targets in 90 percent of the carcinoid/NETs patients they looked at.

The company said their system goes beyond what can be found by DNA sequencing alone and can help

doctors tailor their approaches to specific patients with specific tumor profiles.

Research cites two NETs patients

They cited two specific carcinoid/NETs patients. In one, they found alterations in the expressions of three proteins and one somatic mutation. Identifying these variations, they said, identified an effective treatment for that patient.

In the second they found an alteration in a protein not generally measured in carcinoid/NETs patients that proved relevant to treatment.

CAPTAM study still the research headliner

The other major event of the last few months, was Dr. Robert Fine’s work with the CAPTAM drug combination we reported on last month. While his results were only an interim report, the fact the pairing is moving into a small Phase III trial is important.

I have read some patient accounts from England in recent days about their use of CAPTAM. Those stories make it appear that CAPTAM may be getting regular use in Europe, though I have only seen anecdotal evidence of that to this point. I’ll keep digging in the hope of finding out more.

Patients supporting patients

Spend a few days browsing the online carcinoid/NETs patient support groups on Facebook and you will come away with two impressions. The first is that there is no more caring group of human beings anywhere. There are many such groups for other forms of cancer and for those of us buried in grief that are as supportive and familial as the various ‘noid groups I spent time with over the last week. But none exceed them.

…discoveries will mean nothing if no one hears about them…

If you have carcinoid/NETs you need to become a member of one or more of these groups. I wish they had existed–or that we had been able to find them–when Jane was diagnosed. It might have made our lives a bit easier to have someone to share our agony with who really knew and understood what we were feeling. Don’t misunderstand me: We had great doctors and nurses and wonderful friends. But a carcinoid diagnosis is like the experience of grief–a thing only those who are going through it fully understand.

Support from doctors uneven

The other thing I came away with, though, was less positive. Because of the amount of time I spend with researchers and doctors and patients here in New England–where we have first class facilities and some of the most knowledgeable people in the world about this disease–I sometimes lose track of how desperate and frustrating this disease is for the vast majority of patients out there.

…only those who are going through it fully understand.

There are pockets where you can get the necessary expertise you need, but outside those pockets is a vast wasteland inhabited by oncologists who are not up on the current thinking–at best. At worst, they have so little idea about the disease that they don’t even perceive it as a cancer.

Horror stories from the front lines

On one site I visited to try to generate the list of patients and deceased for this year’s Walking with Jane Marathon Walk T-shirt, a recently diagnosed woman was stunned to learn from that post that you can die from carcinoid/NETs. Her oncologist had apparently told her it was a “manageable disease” like diabetes–and not even potentially fatal.

…outside those pockets is a vast wasteland…

Another was told that despite high readings on the Chromagranin A and 5-HIAA urine tests, that if a set of scans came back negative, they could “lay the carcinoid thing to rest.” Another was told to ignore anything learned on the Internet, even things posted by Dr. Eugene Woltering, one of the leaders in the field.

Patients provided better information than doctors

People had routinely been told by oncologists that carcinoid was not really dangerous, that it grew very slowly, that it was unlikely to spread, that there was no need for follow-up exams beyond a few years after successful surgery, that the disease could be managed, that they would live fully normal lives, that the tumors were benign.

Her oncologist…told her it was a ‘manageable disease’

I’m a layman–an English major, no less. I have no special training in medicine beyond having read the various studies done on carcinoid/NETs in recent years. I am certainly not an oncologist of any kind. The only truth in that laundry list of claims above is that most carcinoid/NETs tumors grow relatively slowly–and that slow growth is not a benefit because it makes it harder to kill.

None of these patients are stupid. They listen, as anyone would, to what a person they perceive as an expert in the field tells them. The problem lies with the doctors, not with the patients.

We need to do more to educate doctors

I would think an oncologist, confronted with carcinoid/NETs for the first time, would seek out the latest knowledge on the disease and base her/his actions on that knowledge. Apparently, that does not happen as often as it should.

None of these patients are stupid.

There is great frustration and anger in the carcinoid/NETs patient community. Yet there is deep and abiding patience and love there as well. Many of these patients deserve better than they are getting. My time with them reminded me just how important the education portion of this mission is. Yes, we need to continue to support the research side, but our discoveries will mean nothing if no one hears about them–or acts on them.

Editor’s Note: All these support groups are private groups on Facebook. They are open only to patients and their immediate families.

Two kinds of tears

Sad tears never came easily when Jane was alive. When you live in joy there is rarely anything that can make you that unhappy. Since her death, happy tears have come rarely. When you live in sorrow there is rarely anything that can make you that happy.

…sometimes I hear news that reduces me to joyous tears.

I have cried twice today–once for sorrow and once for happiness. That is the nature of the Extra Mile Jimmy Fund Marathon Walk Brunch, which I attended for the third time this morning. It is an event mixed of equal parts joy and sorrow for me, but this morning especially so.

Mother on a mission

Each year, one of the speeches at the event comes from either a patient or a family member. This morning, a woman who lost her son to cancer spoke. She lost her high school age son to cancer two years ago. I have seen parents crippled forever by that kind of loss. She, however, refuses to let her own pain stop her from helping others.

 Since her death, happy tears have come rarely.

Her first Jimmy Fund Walk started at Dana-Farber. She walked those three miles with her son. They crossed the finish line together. A year later, she formed a two-person team with another mother whose daughter was fighting cancer. They called their team MOM, for Moms on a Mission. They walked 26.2 miles each and met their son and daughter at the finish line.

A new vocation

Her son’s struggle and death put her on a new mission that has sent her back to school to become a medical interpreter, so that parents who do not speak English can have an advocate to explain what is going on to them. She said, “Cancer speaks every language.” My eyes were already soggy by then.

They crossed the finish line together.

I managed not to cry when a woman I met by chance a year ago at the Brunch stopped by our table. We had talked about some symptoms she was experiencing at the time, and I had urged her to see her doctor about them. This fall they removed a substantial growth from her intestines.

Tears of joy

She has a son who was diagnosed several years ago with cancer. She had begun walking when he was diagnosed. Now she has two reasons to walk–and two reasons to fight. Tomorrow, she will get the test results that will likely tell her they got it all.

Cancer speaks every language.

It was only after I got home I gave in to the emotions I felt as we talked. I wept and cried and let the tears flow down my face.

Accepting the pain of joy

Joy is an emotion I fear more than sorrow these days. It rips through me in unaccustomed ways and takes me places I am not yet ready for my soul to go. Sorrow is a comfortable old shoe. Joy aches like a blister that I fear will break too easily into new pain.

Now she has two reasons to walk…

But I will take the joy, regardless of that fear–or any pain that may come with it. She has won more time to live, to enjoy her son, to do the things she loves. I am jealous of that–jealous of the time she has won that Jane and I did not get–but that jealousy also feeds my joy at her survival: We have stolen another life from cancer’s maw.

The potential joy of walking

People ask why I do what I do: why I walk through the night at Relay, why I put in the miles and miles of training, why I trek from Hopkinton to Boston for the Jimmy Fund, why I write this blog, present at that health fair, pursue knowledge of cancer down every blind alley.

Joy aches like a blister…

Every step, every chance meeting, every pamphlet or letter or piece of writing, may raise the dollar that saves a life or spur a thought that leads to some treatment or get some person who thinks everything is fine to ask the niggling question a doctor needs to hear. Each day is an opportunity to push the walls of death back another micron and gain another bit of life for someone without other hope.

And sometimes I hear news that reduces me to joyous tears. You come, too.